Summary
Surgical and postmortem specimens of lymphoreticular tissue (organized and diffuse) from three patients with Steinbrinck-Chediak-Higashi syndrome were investigated by the light and electron microscope. Material before and after cancer chemotherapy was available. An extensive generalized hyperplasia of lymphoreticular tissues was observed in which several cell lines participated, including lymphocytes, histiocytes, and reticulum cells. Although this hyperplasia resembled neoplasia, histologic and cytologic details suggested it was only a reactive process. Therapy with prednisone and vincristine reduced the well differentiated cells in the lymphoreticular tissues, resulting in a more anaplastic population of immature lymphoid and reticulum cells. It is postulated that proliferation of the lymphoid and histiocytic stem cells is a reaction to the accumulation of materials normally degraded by phagocytes. This proliferation initially may be compensatory, but owing to persistent stimulation by nondegraded foreign materials it progresses to simulate neoplasia.
Zusammenfassung
Organisiertes und nicht organisiertes lymphoretikuläres Gewebe von 3 Patienten mit Steinbrinck-Chediak-Higashi Syndrome vor und nach Behandlung mit Prednison und Vincristin wurden licht- und elektronenoptisch untersucht (Biopsie- und Autopsiematerial). Dabei fand sich eine generalisierte Hyperplasie dieser Gewebe unter Beteiligung verschiedener Zellreihen, wie z. B. Lymphocyten, Histiocyten und Reticulumzellen. Obgleich das Ausmaß dieser Hyperplasie dem einer Neoplasie gleichkam, ähnelten histologische und cytologische Details eher einem reaktiven Prozeß. Behandlung mit Prednison und Vincristin war gefolgt von einer Abnahme differenzierter Zellen, so daß eine mehr anaplastische Population von Lymphoidzellen und Reticulumzellen das Bild beherrschte. Es wurde die Möglichkeit diskutiert, daß die Anhäufung von normalerweise in Phagocyten abgebauten Materials eine Proliferation von Lymphoidzellen und histiocytären Stammzellen anreize. Diese Zellproliferation, anfänglich kompensatorisch, mag wegen persistierender Stimulierung ein Ausmaß annehmen, das von dem einer Neoplasie nicht zu unterscheiden ist. Chemotherapie kann zu diesem anaplastischen Bild infolge Zerstörung differenzierter Zellen und Herabsetzung der immunologischen Reaktionsfähigkeit weiter beisteuern.
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We are indebted to Mr. Herman Mitchelich for technical assistance and reproduction of the electron micrographs and to Mr. Ralph Isenburg for photography.
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Krüger, G.R.F., Bedoya, V. & Grimley, P.M. Lymphoreticular tissue lesions in Steinbrinck-Chediak-Higashi syndrome. Virchows Arch. Abt. A Path. Anat. 353, 273–288 (1971). https://doi.org/10.1007/BF00549041
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DOI: https://doi.org/10.1007/BF00549041